CTICasesAdvanced Pulmonary Langerhans Cell Histiocytosis with an Unusual Vascular Finding
Imaging findings
A heavy smoker with shortness of breath and hypoxia had a radiograph with a reticular-appearing pattern but preserved lung volumes and no true architectural distortion, and CT showed a very advanced cystic/nodular pattern with relatively robust vasculature (too much preserved architecture to be pure emphysema), classic for pulmonary Langerhans cell histiocytosis (PLCH). An unusual solitary tortuous vessel followed a bronchus into a hyperinflated middle lobe segment that was relatively spared from the PLCH; no definite large draining vein could be identified to classify it as an arteriovenous malformation, and it was thought to possibly reflect an artery altered by the patient's severe pulmonary hypertension (confirmed by right heart catheterization) rather than a true AVM.
Key takeaways
Very advanced pulmonary Langerhans cell histiocytosis in a heavy smoker shows extensive cysts with relatively preserved, robust pulmonary vasculature (distinguishing it from simple emphysema) and can be complicated by disproportionately severe pulmonary hypertension out of proportion to the degree of parenchymal destruction; an isolated tortuous vessel following an airway into a relatively spared, hyperinflated segment without an identifiable large draining vein illustrates a diagnostic dilemma between an arteriovenous malformation and an artery altered by chronic pulmonary hypertension.
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